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Observational study to preliminarily characterize the audiological profile of chinese children with williams syndrome
Jiao Zhang1,2,3, Minghui Zhao1, Wei Shi1
1College of Otolaryngology Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China.
Insights
Hearing loss in Chinese children with Williams syndrome (WS) can begin in infancy. Early diagnosis and management are crucial for affected children to achieve near-normal school life, guiding future clinical care.
Area of Science:
- Genetics and Audiology
- Pediatric Health
- Developmental Disorders
Background:
- Williams syndrome (WS) is a rare genetic disorder with variable audiological manifestations.
- Understanding the hearing profiles in diverse populations is critical for effective management.
- Previous studies on WS hearing loss often focused on Western populations.
Purpose of the Study:
- To investigate the audiological characteristics and hearing loss management in Chinese children with Williams syndrome.
- To establish early diagnostic and management references for WS-related hearing impairment in a multicultural context.
- To highlight the importance of early audiological screening in infants with WS.
Main Methods:
- Recruitment of families with Williams syndrome patients from a newborn hearing loss cohort study.
- Performance of comprehensive audiological tests for affected children.
- Review of the global hearing loss phenotype in Williams syndrome.
Main Results:
- Identification of moderately severe sensorineural or conductive hearing loss in Chinese children with WS, emerging in infancy.
- Extension of the known onset age of hearing loss in WS to the infancy period.
- Demonstration that early diagnosis, management, and monitoring enable children with WS to achieve normal or near-normal school life.
Conclusions:
- This study presents the first distinct hearing profile for Chinese children with Williams syndrome.
- The findings emphasize the critical need for early and continuous hearing screening and monitoring in individuals with WS.
- The data serve as valuable references for otolaryngologists and pediatricians in managing hearing loss in WS patients.
Purpose:
It is essential to investigate the audiological profiles of Williams syndrome in a multicultural context. This study aims to examine the characteristics and management of hearing loss in Chinese children with Williams syndrome and provide references for future clinical management.
Method:
Between January 2007 and March 2022, families with at least 1 WS patient was recruited from the Newborn Cohort Study of Hearing Loss. Audiological tests were performed, and then appropriate medical management was offered. Furthermore, an overview of the hearing loss phenotype in Williams syndrome in different locations was reviewed.
Results:
A total of two families with at least 1 Williams syndrome patient were recruited from the Newborn Cohort Study of Hearing Loss (ChiCTR2100049765). We identified moderately severe sensorineural or conductive hearing loss that emerged as early as the infancy period in Williams syndrome subjects in Chinese children. Our results extended the reported onset ages of hearing loss in WS from late childhood or early adulthood to the infancy period. We also found that with early diagnosis, proper management, and regular monitoring, children with Williams syndrome could return to a normal or near-normal school life.
Conclusions:
Our study demonstrated the distinct hearing profile in Chinese children with Williams syndrome for the first time. This cohort of WS subjects extends the reported onset ages of hearing loss in WS from late childhood or early adulthood to the infancy period, indicating the importance of clinicians screening and monitoring the hearing status of individuals with WS as early as possible. These data provide references for otolaryngologists and paediatricians to inform the clinical understanding and management of hearing loss in Williams syndrome.
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